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Factor VII deficiency
Alternative namesExtrinsic factor deficiency
DefinitionFactor VII deficiency is an inherited disorder that causes abnormal blood clotting (coagulation), resulting from a deficiency of the plasma protein factor VII.
Causes, incidence, and risk factors
This disorder is caused by an inherited deficiency of factor VII, an important clotting protein. Normal blood coagulation is a complex process involving as many as 20 different plasma proteins, which are known as blood coagulation factors. A series of complex chemical reactions using these factors takes place very rapidly to form an insoluble protein called fibrin that stops bleeding .
When certain coagulation factors are deficient or missing, the chain reaction does not take place normally. In this disorder, bleeding can vary from mild to severe within the same person over time. A history of bleeding may occur in infancy or childhood. Gastrointestinal and central nervous system bleeding can occur.
The risk factor is a family history of bleeding. The incidence is 1 in 500,000.
Signs and tests
TreatmentBleeding episodes can be controlled with normal plasma, concentrates containing factor VII, or recombinant factor VII. During bleeding episodes, frequent treatment is needed because the life span of factor VII is short. Menstrual bleeding can be controlled by the use of oral contraceptives.
Support GroupsThe stress of illness can often be helped by joining a support group where members share common experiences and problems. See hemophilia - resources .
Expectations (prognosis)The probable outcome is good with proper treatment.
Calling your health care providerGo to the emergency room or call the local emergency number (such as 911) if you have severe, unexplained bleeding .
PreventionThis is an inherited disorder. There is no known prevention.
Update Date: 8/18/2003Corey Cutler, M.D., M.P.H., F.R.C.P.C, Department of Medical Oncology, Dana-Farber Cancer Institute; Instructor in Medicine, Harvard University, Boston, MA. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT